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Nocturnal Paroxysmal Dystonia

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Abstract

Nocturnal paroxysmal dystonia (NPD) along with paroxysmal arousals (PAs) and episodic nocturnal wanderings (ENW) are considered the three characteristic manifestations of nocturnal frontal lobe epilepsy (NFLE). Episodes of NPD begin as paroxysmal arousals, but are subsequently associated with more complex movements including bipedal automatisms, rhythmic twisting movements of the trunk and pelvis, vocalization, and tonic or dystonic posturing lasting less than a few minutes. The epileptic versus nonepileptic origin of NPD has been extensively debated. A network including frontal and possible extrafrontal limbic structures is involved in the genesis of the complex epileptic manifestations of NFLE. Recently, we have started recognizing the co-occurrence of NFLE with parasomnias. The observation that similar motor features can accompany both arousal disorder parasomnias and seizures, including those of NFLE has led to the hypothesis that both phenomena may indicate a release of normal neocortical inhibition of central pattern generators in mesencephalon, pons, and/or spinal cord. The predominant source of confusion is episodes characterized by sudden arousal from sleep with motor activity and often dramatic or bizarre behaviors. As such, the disorders to be considered are the NREM arousal parasomnias, rapid eye movement (REM) sleep behavior disorder, and nocturnal panic attacks as they may present in the similar fashion. A small dose of carbamazepine treatment is effective in 70 % of the patients, whereas a third of the patients, commonly with a high seizure frequency, are drug resistant. In patients with NFLE, use of individually tailored antiepileptic drug (AED) dosing, that is, higher doses of AEDs in the evening, may be helpful.

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Authors and Affiliations

  1. Division of Pediatric Neurology, Department of Pediatrics, University Hospital of Patras, Rio, 26504, Patras, Greece

    Joseph Kaleyias MD, PhD

  2. Jenny Lind Children’s Department, Norfolk and Norwich University Hospital, Colney Lane, NR4 7UY, Norwich, UK

    Ruchi Arora MBBS, DTCH, MRCPCH

  3. Center for Pediatric Sleep Disorders, Department of Neurology, Boston Children’s Hospital, Harvard Medical School, 02115, Boston, MA, USA

    Sanjeev V. Kothare MD

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  1. Joseph Kaleyias MD, PhD
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  2. Ruchi Arora MBBS, DTCH, MRCPCH
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  3. Sanjeev V. Kothare MD
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Correspondence to Joseph Kaleyias MD, PhD .

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Editors and Affiliations

  1. Harvard Medical School, Associate Professor, Department of Neuro, Boston Children's Hospital, Longwood Avenue 300, Boston, 02115, Massachusetts, USA

    Sanjeev V. Kothare

  2. Feinberg School of Medicine, Alexian Brothers Medical Center, Northwestern University, Biesterfield Road 800, Elk Grove Village, 60007, Illinois, USA

    Anna Ivanenko

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Kaleyias, J., Arora, R., Kothare, S. (2013). Nocturnal Paroxysmal Dystonia. In: Kothare, S., Ivanenko, A. (eds) Parasomnias. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-7627-6_17

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